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IMMUNOLOGY Flashcards

1
Q

How many % of anaphylactic episodes is uniphasic and biphasic?

A

a. 80-90% uniphasic

b. 10-20% biphasic

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2
Q

Most common presentations of anaphylaxis (>90% of cases)

A

Cutaneous manifestation

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3
Q

Severe hymenoptera-induced anaphylaxis can be a presenting feature of what underlying condition?

A

Systemic mastocytosis

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4
Q

What is the mechanism involved when a repeated exposure to an allergen induces anaphylaxis?

A

IgE – mediated (usually with antibiotics and chemotherapy)

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5
Q

Paclitaxel-induced anaphylaxis: IgE or non-IgE-mediated?

A

Non-IgE

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6
Q

Radiocontrast-induced anaphylaxis: IgE or non-IgE-mediated?

A

Non-IgE

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7
Q

Vancomycin-induced anaphylaxis: IgE or non-IgE-mediated?

A

Non-IgE

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8
Q

Opiates-induced anaphylaxis: IgE or non-IgE-mediated?

A

Non-IgE

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9
Q

NSAIDs-induced anaphylaxis: IgE or non-IgE-mediated?

A

Non-IgE

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10
Q

Onset of symptoms and signs of anaphylaxis occurs within seconds to minutes after the trigger, except for:

A

Delayed anaphylaxis to meats in alpha-gal sensitized patients

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11
Q

Most obvious biomarker of anaphylaxis:

A

Histamine (but with extremely short half-life)

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12
Q

More practical and useful biomarker of anaphylaxis

A

Serum tryptase

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13
Q

Treatment of anaphylaxis and dose:

A

Epinephrine – 0.3-0.5 mL of 1:1000 (1 mg/mL) IM with repeated doses at 5-20 min intervals as needed for a severe reaction

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14
Q

Body posture that may lead to “empty heart syndrome” in anaphylaxis

A

Upright or sitting posture

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15
Q

Recommended body position before receiving epinephrine in anaphylaxis

A

Supine position

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16
Q

Simplest, most straightforward approach to long-term management of a patient with a history of anaphylaxis

A

Avoidance

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17
Q

Study stating that early introduction of peanut protein to the diet of high-risk infants can prevent development of most (80% or more) peanut allergy

A

Learning Early About Peanut Allergy (LEAP) study

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18
Q

Premedication regimens for radiocontrast allergy with doses and timing (2):

A

a. Prednisone 0.5 mg/kg at 13, 6, and 1 h prior to contrast administration
b. Diphenhydramine – 25 mg 1 h prior to contrast

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19
Q

Management of flushing reactions from vancomycin (2):

A

a. Antihistamine premedication

b. Downtitrate infusion rate

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20
Q

Refers to a group of autoantibody-mediated intraepidermal blistering diseases characterized by loss of cohesion between epidermal cells

A

Pemphigus

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21
Q

Loss of cohesion between epidermal cells is a process termed

A

Acantholysis

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22
Q

Manual pressure to the skin of pemphigus patients may elicit the separation of the epidermis. This is called

A

Nikolsky’s sign

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23
Q

Mucocutaneous blistering disease that predominantly occurs in patients >40 years of age and typically begins on mucosal surfaces and often progresses to involve the skin.
This disease is characterized by fragile, flaccid blisters that rupture to produce extensive denudation of mucous membranes and skin

A

Pemphigus vulgaris (PV)

The mouth, scalp, face, neck, axilla, groin, and trunk are typically involved

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24
Q

Crusts and shallow erosions on scalp, central face, upper chest, and back

A

Pemphigus foliaceus

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25
Q

Painful stomatitis with papulosquamous or lichenoid eruptions that may progress to blisters

A

Paraneoplastic pemphigus

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26
Q

Large tense blisters on flexor surfaces and trunk with histologic finding of subepidermal blister with eosinophil-rich infiltrate

A

Bullous pemphigoid

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27
Q

Pruritic, urticarial plaques rimmed by vesicles and bullae on the trunk and extremities with the histologic findings of teardrop-shaped, subepidermal blisters in dermal papillae; eosinophil- rich infiltrate

A

Pemphigoid gestationis

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28
Q

Extremely pruritic small papules and vesicles on elbows, knees, buttocks, and posterior neck with histology of subepidermal blister with neutrophils in dermal papillae and with granular deposits of IgA in dermal papillae

A

Dermatitis herpetiformis

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29
Q

Blisters, erosions, scars, and milia on sites exposed to trauma; widespread, inflammatory, tense blisters may be seen initially

A

Epidermolysis bullosa acquisita

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30
Q

Dermatomyositis often involves the hands as erythematous flat-topped papules over the knuckles. What do you call this papules?

A

Gottron’s papules

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31
Q

Patients with Pemphigus vulgaris have IgG autoantibodies to

A

desmogleins (Dsgs)

transmembrane desmosomal glycoproteins that belong to the cadherin family of calcium-dependent adhesion molecules

early PV – Dsg3
Advanced PV – Dsg3 and Dsg1

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32
Q

Bad prognostic factors for pemphigus vulgaris

A
  1. Advanced age
  2. Widespread involvement
  3. Requirement for high doses of glucocorticoids
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33
Q

mainstay of treatment for pemphigus vulgaris

A

Systemic glucocorticoids

moderate to severe PV - prednisone at 1 mg/kg/day

may be combined with immunosuppressive agents such as azathioprine, mycophenolate mofetil, rituximab, or cyclophosphamide

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34
Q

Treatment of severe or progressive pemphigus vulgaris

A

Plasmaperesis or IV Ig

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35
Q

Pemphigus wherein acantholytic blisters are located high within the epidermis, usually just beneath the stratum corneum

A

Pemphigus foliaceus

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36
Q

Which is more superficial: Pemphigus foliaceus or vulgaris?

A

Pemphigus foliaceus

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37
Q

In terms of mucous membrane involvement, differentiate Pemphigus foliaceus and vulgaris

A

Foliaceus – mucous membrane is almost always spared

Vulgaris - mucous membrane is usually involved

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38
Q

Mild cases of pemphigus foliaceus can resemble

A

severe seborrheic dermatitis

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39
Q

Direct immunofluorescence microscopy of perilesional skin ofpemphigus vulgaris and foliaceus demonstrates ___ on the surface of keratinocytes

A

IgG

40
Q

In pemphigus foliaceus, autoantibodies are directed against

A

Dsg1

41
Q

Although pemphigus has been associated with several autoimmune diseases, its association with _____ and/or ______ is particularly notable

A

Thymoma

myasthenia gravis

42
Q

T or F. Drug-induced pemphigus usually resembles PV rather than PF

A

False. drug-induced pemphigus usually resembles PF rather than PV

43
Q

T or F. Pemphigus foliaceus is generally a less severe disease than PV and usually carries a better prognosis

A

True

44
Q

An autoimmune acantholytic mucocutaneous disease associated with an occult or confirmed neoplasm

A

Paraneoplastic pemphigus (PNP)

45
Q

The predominant neoplasms associated with paraneoplastic pemphigus are (6)

A
  1. non-Hodgkin’s lymphoma
  2. Chronic lymphocytic leukemia
  3. Thymoma
  4. Spindle cell tumor
  5. Waldenström’s macroglobulinemia
  6. Castleman’s disease (children with PNP)
46
Q

In addition to severe skin lesions, many patients with paraneoplastic pemphigus develop life-threatening

A

bronchiolitis obliterans

47
Q

A polymorphic autoimmune subepidermal blistering disease usually seen in the elderly that evolve from urticarial plaques to tense blisters on either normal-appearing or erythematous skin

A

Bullous pemphigoid

The lesions are usually distributed over the lower abdomen, groin, and flexor surface of the extremities

48
Q

The major histocompatibility complex class II allele _______ is prevalent in patients with bullous pemphigoid

A

HLA-DQβ1*0301

49
Q

Direct immunofluorescence microscopy of normal-appearing perilesional skin from patients with this condition shows linear deposits of IgG and/ or C3 in the epidermal basement membrane

A

Bullous pemphigoid

50
Q

An alternative immunofluorescence microscopy test substrate used to distinguish circulating IgG autoantibodies to the basement membrane in patients with bullous pemphigoid from those in patients with similar, yet different, subepidermal blistering diseases

A

1 M NaCl split skin

51
Q

mainstay of treatment of bullous pemphigoid

A

Systemic glucocorticoids

52
Q

A rare, nonviral, subepidermal blistering disease of pregnancy and the puerperium

A

Pemphigoid gestationis

any trimester of pregnancy or present shortly after delivery

53
Q

Immunologically-mediated skin condition that is almost always extremely pruritic

A

Pemphigoid gestationis

54
Q

Linear deposits of C3 in the epidermal basement membrane is an immunopathologic hallmark of this disorder

A

Pemphigoid gestationis

55
Q

A subepidermal bullous disease that resembles pemphigoid gestationis clinically, histologically, and immunopathologically

A

Bullous pemphigoid

56
Q

An intensely pruritic, papulovesicular skin disease characterized by lesions symmetrically distributed over extensor surfaces (i.e., elbows, knees, buttocks, back, scalp, and posterior neck)

A

Dermatitis herpetiformis

pruritus has a distinctive burning or stinging component

57
Q

Almost all dermatitis patients have associated (usually subclinical) GI condition. This is the

A

gluten-sensitive enteropathy

58
Q

The major histocompatibility complex class II allele that is present in >90% or dermatitis herpetiformis (2)

A

HLA-B8/DRw3 and HLA-DQw2

59
Q

Subepidermal blistering disorder that is confirmed by direct immunofluorescence microscopy of normal-appearing perilesional skin and granular deposits of IgA (with or without complement components) in the papillary dermis and along the epidermal basement membrane zone

A

Dermatitis herpetiformis

60
Q

If maintained, this diet alone may control the skin disease in dermatitis herpetiformis and eventuate in clearance of IgA deposits from these patients’ epidermal basement membrane zones

A

Gluten-free diet

61
Q

Mainstay of treatment of dermatitis herpetiformis

A

Dapsone

Patients respond rapidly (24–48 h) to dapsone (50–200 mg/d), but require careful pretreatment evaluation and close follow-up to ensure that complications are avoided or controlled

62
Q

All patients taking dapsone at _____ will have some _____ and______ , which are expected pharmacologic side effects of this agent

A

> 100 mg/d

Hemolysis
Methemoglobinemia

63
Q

Difference of linear IgA disease from dermatitis herpetiformis (2)

A

Do not have increased frequency of HLA-B8/DRw3 haplotype or an associated gluten-sensitive enteropathy

64
Q

Treatment of linear IgA disease

A

Dapsone

65
Q

A rare, noninherited, polymorphic, chronic, subepidermal blistering disease characterized by blisters on noninflamed skin, atrophic scars, milia, nail dystrophy, and oral lesions

A

Epidermolysis bullosa acquisita

66
Q

Subepidermal blistering disease that is considered as a mechanobullous disease

A

Epidermolysis bullosa acquisita

67
Q

The major histocompatibility complex class II allele that is found in epidermolysis bullosa acquisita

A

HLA-DR2 haplotype

68
Q

Epidermolysis bullosa acquisita is associated with what GI condition

A

IBD (esp. Crohn’s disease)

69
Q

Subepidermal blistering disease that is largely resistant to treatment

A

chronic, noninflammatory form of epidermolysis bullosa acquisita

although some patients may respond to cyclosporine, azathioprine, IVIg, or rituximab

inflammatory form may respond to systemic glucocorticoids

70
Q

A rare, acquired, subepithelial immunobullous disease that commonly involve the oral mucosa (esp. the gingiva) and conjunctiva that is characterized by erosive lesions of mucous membranes and skin that result in scarring of at least some sites of involvement

A

Mucous membrane pemphigoid

typically a chronic and progressive disorder

71
Q

Studies suggest that mucous membrane pemphigoid

patients with autoantibodies to ____ have an increased relative risk for cancer

A

laminin-332

72
Q

Heliotrope erythema is seen in what condition

A

Dermatomyositis

73
Q

purple-red discoloration of the upper eyelids, sometimes associated with scaling and periorbital edema

A

“heliotrope” erythema

74
Q

The most common cutaneous manifestation of dermatomyositis

A

“heliotrope” erythema

75
Q

Pathognomonic for dermatomyositis

A

Gottron’s papules

These lesions can be contrasted with the erythema and scaling on the dorsum of the fingers that spares the skin over the interphalangeal joints of some SLE patients

76
Q

Thin violaceous papules and plaques on the elbows and knees of patients with dermatomyositis are referred to as

A

Gottron’s sign

77
Q

T or F. Poikiloderma is a common feature seen SLE, scleroderma and dermatomyositis

A

False. Poikiloderma is rare in both SLE and scleroderma and thus can serve as a clinical sign that distinguishes dermatomyositis from these two diseases

78
Q

Acute cutaneous LE is characterized by

A

erythema of the nose and malar eminences in a “butterfly” distribution

79
Q

Characterized by a widespread photosensitive, nonscarring eruption that may present as a papulosquamous eruption that resembles psoriasis or as annular polycyclic lesions

A

Subacute cutaneous lupus erythematosus (SCLE)

80
Q

Autoantibodies in subacute cutaneous lupus erythematosus (SCLE)

A

Ant-Ro

81
Q

Characterized by discrete lesions, most often found on the face, scalp, and/or external ears and present as erythematous papules or plaques with a thick, adherent scale that occludes hair follicles (follicular plugging)

A

Discoid lupus erythematosus

82
Q

In this condition, when a scale that occludes hair follicles is removed , its underside shows small excrescences that correlate with the openings of hair follicles (so-called “carpet tacking”)

A

Discoid lupus erythematosus

A finding relatively specific for DLE

83
Q

The skin changes of scleroderma usually begin on the (5)

A

fingers, hands, toes, feet, and face

84
Q

Sclerodactyly is seen in

A

Scleroderma

85
Q

The fingers may shrink and become sausage-shaped, and, because the fingernails are usually unaffected, they may curve over the end of the fingertips. This is seen in

A

Scleroderma

86
Q

Facial involvement of this condition includes a smooth, unwrinkled brow, taut skin over the nose, shrinkage of tissue around the mouth, and perioral
radial furrowing

A

Scleroderma

87
Q

Cold-induced blanching, cyanosis, and reactive hyperemia that is documented in almost all patients with scleroderma and can precede development of the disease by many years

A

Raynaud’s phenomenon

88
Q

CREST syndrome is combination of

A 
Calcinosis cutis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectases
89
Q

Autoantibodies that is seen in a very high percentage of patients with CREST syndrome but in only a small minority of patients with scleroderma

A

Anti-centromere

90
Q

Characterized by localized thickening and sclerosis of skin that is usually located in the trunk

A

Morphea

91
Q

T or F. Morphea, unlike scleroderma, is usually quite resistant to therapy

A

False. Scleroderma and morphea are usually quite resistant to therapy

Treatment options for early, rapidly progressive disease include phototherapy (UVA1 [ultraviolet A1 irradiation] or PUVA [psoralens + ultraviolet A irradiation]) or methotrexate (15–20 mg/week) alone or in combination with daily glucocorticoids

92
Q

A clinical entity that can sometimes be confused with scleroderma and is characterized by a sudden onset of swelling, induration, and erythema of the extremities, frequently following significant physical exertion

A

Diffuse fasciitis with eosinophilia

93
Q

Indurated skin that usually displays a woody, dimpled, or “pseudocellulite” appearance is seen in what condition

A

Diffuse fasciitis with eosinophilia

94
Q

Contractures in this immunologic condition may occur early secondary to fascial involvement and may also cause muscle groups to be separated and veins to appear depressed. What do you call this sign and what condition?

A

Groove sign.

Diffuse fasciitis with eosinophilia

95
Q

A multisystem disorder with epidemic numbers of cases reported in 1989 and linked to ingestion of l-tryptophan manufactured by a single company in Japan, characterized by debilitating myalgias and absolute eosinophilia in association with varying combinations of arthralgias, pulmonary symptoms, and peripheral edema

A

eosinophilia-myalgia syndrome

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